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Monday, February 14, 2011

The founder of CHD-UK shares with us her Personal Story of what it is like to grow up and live with a CHD.

An Overview to the main story:I am a miracle to be alive especially when as I was born in early 70's with Transposition of the Great Arteries and without a hole, I needed to be born with a atrial septal defect as well for the blood flow but I wasn't. However, they operated on me by giving me a balloon sepostmy and then I had major open-heart surgery 2 years later (the Mustard procedure - This operation is still performed but ONLY in certain situations where the Switch is not suitable). I was 2 years and 2 weeks old when I underwent this surgery. There was no information or awareness about CHD then and there still isn't. Now, there is a growing population of adults with CHD which did not exist 30 years ago. We are called GUCH in the UK but I prefer to be called an adult with CHD. I am one of the first generation of survivors and I don't know how long I will live for and whether I will get any more side effects - There is no set rules for what can happen with deterioration with Transposition of the Great Arteries for my generation, one person may need a heart transplant, another will need a valve transplant and another may only need a pacemaker.....

Introduction of myself
I am adult with CHD, I am currently in my late 30's and I grew up in Gloucestershire, lived in London and I now currently live in Spain where I teach English to the Spanish. I have one younger sister who does not have CHD and I am still undecided about having children because all through growing up I was told I could not have children but then five years ago I find out that I can from my Specialist. I have been monitored all my life and I will be for the rest of my life.

I belong to the first generation of congenital heart disease survivors. In the UK we are called GUCH but in America it is called ACCA.

How and why did I survive to become a GUCH/Adult CHD'er?
I will now give you a bit more detail about what I was born with. The congenital heart disease it should be called defect I was born with is what is medically known as Transposition of the Great Arteries. I had several operations before I was 2.5 years old, these were the balloon septostomy in 1973 and The Mustard Operation (open-heart surgery) in 1973 performed by a Dr Besley (the arterial switch is now the preferred operation). What is Transposition of the Great Arteries ? What is a balloon septostomy? What is the Mustard Operation?

Babies born with Transposition of the great arteries are known as blue babies due to the insufficient oxygen circulating in their bodies. Transposition of the great arteries is where the blood from the lungs flows back to the lungs and blood from the body flows back to the body. The two major arteries coming out of the heart, the aorta and the pulmonary artery are connected to the wrong chambers. Such babies look blue because insufficient oxygen is circulating in their bodies.

A balloon septostomy is normally a palliative procedure used to prepare a patient for, or sustain them until a corrective surgery can be performed. At this time the ASD is closed using either sutures or cardiac patch, depending on the size and/or nature of the opening.

The balloon septostomy is the widening of a foramen ovale, patent foramen ovale (PFO), or atrial septal defect (ASD) via cardiac catherisation (heart cath) using a balloon catheter. This procedure allows a greater amount of oxygenated blood to enter the systemic circulation in some cases of cyanotic congenital heart disease (CHD)

After the catheter is inserted, the deflated balloon catheter is passed from the right atrium through the foramen ovale, PFO or ASD into the left atrium, it is then inflated and pulled back through to the right atrium, thereby enlarging the opening and allowing greater amounts of blood to pass through it. The resulting man-made opening is one of many forms of shunting, and is often referred to as an ASD.
The Mustard procedure restores the circulation but reverses the direction of the blood flow in the heart. In a normal heart, de-oxygenated, blue, blood is pumped into the lungs via the right ventricle. Then it is distributed throughout the body via the left ventricle. In the Mustard procedure, blood is pumped to the lungs via the left ventricle and disseminated throughout the body via the right ventricle.

But the right ventricle is not the optimal shape to support the high pressure work performed in a normal heart by the left ventricle. Over a period of 20 or 30 years the right ventricle may begin to fail.
'Was it tough growing up with CHD? Has CHD held me back? How does CHD influence my daily life now?' To the last question, I asked my boyfriend his opinion because I just couldn’t think and his reply was “you think everyone is against you and that you have to fight a battle all time as in "you are very defensive and sensitive”. I suppose what he said is all true and that this all comes from fighting my way to live from being born and trying to survive until I had the Mustard Operation and fighting to live after the operation (that was all a battle). I also think that being defensive and sensitive is a reaction to dealing with the bullying I got for having a CHD at secondary school.... this was mainly due to the fact that I had to wear a 24 holtor monitor, which was the size of the brick, in fact, I remember a kid pulling the monitor off me and throwing it over the netball nets. He got into some trouble for doing that especially as the equipment did not belong to me it belonged to the hospital but that was the extreme bullying and only happened once. I was not allowed to do sports half way through the first year of secondary school this was due to a teacher pushing me and not letting me stop when I knew I had enough and now I am too scared to push myself now when I am exercising. I have also heard since that other teachers doing the same to other children with CHD. There is no awareness, we look normal, like anyone in the street – our condition is not staring people in the face, so they think we are making it up!!!!

I have always lived day to day, having fun. My moto is ‘You have only one life, live life with no regrets and always do what you want to do. Me, I have had a second chance at life so I live it to the full and I am very spontaneous lol.

How did my parents cope?
My parents think that it is a miracle that I am here at all and are proud of me no matter what I have done or do. I am not pushed to do anything great, just to have a life. The reason being is due to the fact that back in the 1970’s there was no internet nor was medical advancement that great. My parents had no one to really turn to; they knew no one in their area who had a child with CHD. There is a charity belonging to Bristol NHS Teaching Hospital where I had the operations and I am still monitored but that was it. My mother still raises money for this charity. My parents had a hard time dealing with having a child with CHD but they eventually coped and to my mind it made them a stronger couple. Before I had the operation my parents had to give me tablets to keep me alive but if I was sick, they could not give me another tablet or it would kill me. This scared them and I cannot imagine what they went through. The lack of help and awareness back when I was born has made me think that there should be more awareness in the world but there isn’t. Can you believe it nearly 40 years later there is still a lack of awareness which is why I decided to set up the CHD-UK page on MySpace and the CHD-UK group on Facebook and now this website.

My parents also had to face the fact that they had to move house because where they lived was too cold for me and they always had the heating on. If I was not kept warm I would go blue due to the cold, the heart has to pump harder to keep the circulation flowing and your body automatically protects its vital organs. Even now I still suffer from the cold, my lips, fingers and toes all go blue. I need to keep warm or otherwise my heart will be working too hard just to keep my fingers, toes and lips from going blue.

As I mentioned before I am one of the first generation of survivors also known as GUCH/Adult CHD'er. We are the ones who are now having major or minor problems with our health, some with no insurance causing us increased and unnecessary stress (USA), having to pay for prescriptions for medication that is necessary to keep us well. Having to pay expensive travel insurance just to go abroad, having to pay more for insurance just because we have a CHD. To my mind, we are healthier than the average citizen because we are monitored every year whereas the average citizen isn’t. The government will not do anything about this! In fact, we will be a future drain on governments of all countries. They have no idea yet what happens when CHDs with old repairs (people ages 55 and up) mix with acquired heart disease.

For me, having Transposition of the great arteries the major or minor problems that can arise from the Mustard procedure can vary from arrhythmias to having a heart transplant. I currently have intermittent nodal rhythm and intermittent atrial tachycardia/fibrillation and I still do not know what the future is. But with the old repairs yes they do in a way know what will happen but they are not 100% sure because everyone's problems that arise are different and I also think the age should be from 30+ because my arrhythmias which are caused from an old scaring from the operation started when I was in my early 30s and luckily I still do not have the pacemaker yet, but I will need definitely in the future, but after that I do not know what problems will arise nor do the Specialists.

At the moment I wouldn't change anything in my life, I am who I am, defected heart included – it’s made me who I am today. That is a strong, outgoing, stubborn (had to put that in), independent, thoughtful, caring person that I am today.